Successful perioperative management of factor X deficiency associated with primary amyloidosis

Takabe, Kazuaki

doi:10.1016/j.gassur.2003.10.014

Cited by 29 publications

(16 citation statements)

References 33 publications

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“…While acquired FX deficiency may be eliminated by treating the underlying cause, the congenital form of the disease is lifelong and is among the most severe clotting factor disorders. In general, patients with very low levels of functional FX have a greater tendency to hemorrhage and face a greater risk of life-threatening complications [14, 15]. …”

Section: Discussionmentioning

confidence: 99%

Membranoproliferative glomerulonephritis and a rare bleeding disorder: factor X deficiency

et al. 2010

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Factor X (FX) deficiency is a rare hereditary coagulation disorder. This is the first case report on the association of FX deficiency and membranoproliferative glomerulonephritis (MPGN) type I. The patient, a 17-year-old male, presented with edema, hypertension, and microscopic hematuria, followed by a mild upper respiratory tract infection. Laboratory tests revealed: serum creatinine 1.6 mg/dl, serum albumin 2.80 g/dl, C3 16 mg/dl and proteinuria (1,800 mg/day). The renal biopsy showed MPGN type I. The coagulation profile prior to percutaneous renal biopsy revealed prolonged prothrombin time and activated partial thromboplastin time values. The patient was given fresh frozen plasma and vitamin K before the biopsy. Further evaluation showed the functional activity of FX was 7% of the norm. This case emphasizes the need for routine coagulation screening before percutaneous renal biopsy.

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Section: Discussionmentioning

confidence: 99%

Membranoproliferative glomerulonephritis and a rare bleeding disorder: factor X deficiency

et al. 2010

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“…FIX concentrates like Bebulin TM can be used to treat FX-deficient patients; however, efficiency is unknown [9]. A usual daily requirement can be given in a single dose or 2 divided doses because FX has a long half-life, i.e., 48 hours [12], but more frequent dosing may be needed in AL.…”

Section: Discussionmentioning

confidence: 99%

“…Acquired deficiency of factor X (FX) is the most common coagulation factor deficiency and appears to be secondary to adsorption of FX to amyloid fibrils [1,2]. There is presently insufficient data regarding management of FX deficiency associated with primary amyloidosis [3][4][5][6][7][8][9]. In this report, we discuss the management of acquired FX deficiency.…”

Section: Introductionmentioning

confidence: 99%

Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency

Barker

Altuntaş

Paranjape

et al. 2004

J of Clinical Apheresis

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In patients with rare factor deficiencies, for which no factor concentrates are available, plasma exchange (PE) is an option for raising the desired factor level to approximately 80% for surgery. We report a case of acquired factor X (FX) deficiency due to amyloidosis that required urgent surgical repair of an AV fistula aneurysm. This patient had a FX level of 3% at presentation; after 1.5 volume PE with fresh frozen plasma (FFP), his post-exchange FX was only 5%, indicating rapid adsorption of FX to amyloid fibrils. He was managed successfully with FEIBA during surgery.

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“…Bohrer et al [41] proposed the use of prothrombin complex concentrate (PCC) to avoid perioperative bleeding in patients of AL amyloidosis with acquired factor X deficiency. Some experts have successfully used recombinant human factor VIIa and Bebulin (a PCC that contains the highest concentration of factor X) in patients with amyloidosis, as fresh frozen plasma is often ineffective in controlling bleeding, and PCC has a significant risk of thrombosis when used intraoperatively in such patients [42,51].…”

Section: Subphrenic Abscess/atelectasis and Damage To Adjacentmentioning

confidence: 99%

Splenectomy in plasma cell dyscrasias: A review of the clinical practice

et al. 2006

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Veterans Healthcare System, Little Rock, ArkansasPlasma cell dyscrasias are a group of clinically and biochemically diverse disorders of unknown etiology, characterized by the disproportionate proliferation of one or more clones of B cells, and the presence of a structurally and electrophoretically homogeneous (monoclonal) immunoglobulin or polypeptide subunit in serum or urine. The role of splenectomy in the management of plasma cell dyscrasias has not been well defined. Using MEDLINE, the authors searched the English-language published literature from the year 1970 through September 2005 to determine the indications for splenectomy in plasma cell dyscrasias.A review of the literature in humans and animals supported the idea that the spleen provides a special microenvironment favorable for homing or differentiation of IgM producing B cells, and splenectomy can, at times, lead to remission in Waldenströ m's macroglobulinemia. The other reported reasons for splenectomy in plasma cell dyscrasias are hypersplenismrelated pancytopenia, control of splenic plasmacytomas, and management of a splenic abscess. Splenic infiltration in primary amyloidosis can be an indication for splenectomy, where removal of a large spleen can also reverse an acquired factor X deficiency. Thus, the spleen can be considered a potential target organ for management of plasma cell dyscrasias, and therapeutic success has been achieved with removal of this organ.However, splenectomy can be a potentially morbid procedure in patients with plasma cell dyscrasias, and major postoperative complications include infection, hemorrhage, and thrombosis. Am. J. Hematol. 81:946-954, 2006. V V C 2006 Wiley-Liss, Inc.

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Successful perioperative management of factor X deficiency associated with primary amyloidosis

Cited by 29 publications

References 33 publications

Membranoproliferative glomerulonephritis and a rare bleeding disorder: factor X deficiency

Membranoproliferative glomerulonephritis and a rare bleeding disorder: factor X deficiency

Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency

Splenectomy in plasma cell dyscrasias: A review of the clinical practice

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